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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)

  • Last Modified: 08/15/2014

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Nephroblastomatosis

General Information About Nephroblastomatosis
Standard Treatment Options for Nephroblastomatosis
        Preoperative chemotherapy
        Renal-sparing surgery



General Information About Nephroblastomatosis

Some multifocal nephrogenic rests may become hyperplastic, which may produce a thick rind of blastemal or tubular cells that enlarge the kidney. Nephroblastomatosis may be diagnosed radiographically by magnetic resonance imaging, in which the homogeneity of the hypointense rind-like lesion on contrast-enhanced imaging differentiates it from Wilms tumor. Biopsy often cannot discriminate Wilms tumor from these hyperplastic nephrogenic rests. Differentiation may occur after chemotherapy is administered.

Standard Treatment Options for Nephroblastomatosis

Standard treatment options for nephroblastomatosis include the following:

  1. Preoperative chemotherapy.
  2. Renal-sparing surgery.
Preoperative chemotherapy

Current treatment includes vincristine and dactinomycin until nearly complete resolution, as determined by imaging.

Renal-sparing surgery

Even with treatment (vincristine and dactinomycin), about one-half of children diagnosed with nephroblastomatosis will develop Wilms tumor within 36 months. In a series of 52 patients, 3 patients died of recurrent Wilms tumor.[1] In treated children, as many as one-third of Wilms tumors are anaplastic, probably as a result of selection of chemotherapy-resistant tumors, so early detection is critical. Patients are monitored by imaging at a maximum interval of 3 months, for a minimum of 7 years. Given the high incidence of bilaterality and the subsequent Wilms tumors, renal-sparing surgery is indicated.[1]

These patients are eligible for treatment on the COG-AREN0534 trial with vincristine and dactinomycin.

References
  1. Perlman EJ, Faria P, Soares A, et al.: Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer 46 (2): 203-21, 2006.  [PUBMED Abstract]